PURPOSE: Long-term survivors of biliary atresia (BA) sometimes experience liver dysfunction. We evaluated the prognostic factors for long-term native liver survival (NLS) in BA patients after the Kasai procedure. METHODS: This study included 67 patients with jaundice disappearance after the Kasai procedure performed between 1972 and 1995, and NLS for over 10 years. We retrospectively evaluated the clinical parameters, including the type of BA, age at the Kasai procedure, medical conditions, and treatments. The adjusted odds ratios (aOR) were obtained for 20-year NLS using logistic regression analysis. RESULTS: The median age of the patients at the Kasai procedure was 63 days. Of the 67 study patients, 62 patients (92.5 %) had jaundice-free NLS at the age of 20 years, 4 patients died before the age of 20 years from liver failure, and 1 patient underwent living related liver transplantation. The presence of gastro-esophageal varices requiring endoscopic injection sclerotherapy was a significant factor (aOR 33.8; p = 0.0033), while hypersplenism and cholangitis were not identified as significant factors. CONCLUSIONS: The existence of symptomatic portal hypertension would influence long-term NLS in BA patients after the Kasai procedure. In such patients, accurate evaluation of hepatic function and adequate treatment for sequelae are needed.
PURPOSE: Long-term survivors of biliary atresia (BA) sometimes experience liver dysfunction. We evaluated the prognostic factors for long-term native liver survival (NLS) in BA patients after the Kasai procedure. METHODS: This study included 67 patients with jaundice disappearance after the Kasai procedure performed between 1972 and 1995, and NLS for over 10 years. We retrospectively evaluated the clinical parameters, including the type of BA, age at the Kasai procedure, medical conditions, and treatments. The adjusted odds ratios (aOR) were obtained for 20-year NLS using logistic regression analysis. RESULTS: The median age of the patients at the Kasai procedure was 63 days. Of the 67 study patients, 62 patients (92.5 %) had jaundice-free NLS at the age of 20 years, 4 patients died before the age of 20 years from liver failure, and 1 patient underwent living related liver transplantation. The presence of gastro-esophageal varices requiring endoscopic injection sclerotherapy was a significant factor (aOR 33.8; p = 0.0033), while hypersplenism and cholangitis were not identified as significant factors. CONCLUSIONS: The existence of symptomatic portal hypertension would influence long-term NLS in BA patients after the Kasai procedure. In such patients, accurate evaluation of hepatic function and adequate treatment for sequelae are needed.
Authors: Vicky Lee Ng; Barbara H Haber; John C Magee; Alexander Miethke; Karen F Murray; Sonia Michail; Saul J Karpen; Nanda Kerkar; Jean P Molleston; Rene Romero; Philip Rosenthal; Kathleen B Schwarz; Benjamin L Shneider; Yumirle P Turmelle; Estella M Alonso; Averell H Sherker; Ronald J Sokol Journal: J Pediatr Date: 2014-07-09 Impact factor: 4.406
Authors: Maria Hukkinen; Jouko Lohi; Päivi Heikkilä; Reetta Kivisaari; Timo Jahnukainen; Hannu Jalanko; Mikko P Pakarinen Journal: Hepatol Commun Date: 2019-01-09
Authors: Veena Venkat; Vicky L Ng; John C Magee; Wen Ye; Kieran Hawthorne; Sanjiv Harpavat; Jean P Molleston; Karen F Murray; Kasper S Wang; Nisreen Soufi; Lee M Bass; Estella M Alonso; Jorge A Bezerra; M Kyle Jensen; Binita M Kamath; Kathleen M Loomes; Cara L Mack; Philip Rosenthal; Benjamin L Shneider; Robert H Squires; Ronald J Sokol; Saul J Karpen Journal: Hepatol Commun Date: 2020-10-03